1. PATOLOGIE TUMORALI ENDOCANALARI
Dott.ssa Anna Pichiecchio
U.O. Neuroradiologia
Istituto Neurologico IRCCS Fondazione C. Mondino, Pavia

2. Classification based on their location
SPINAL TUMORS
Uncommon lesions
Classification based on their location
EXTRADURAL (60% of all spinal tumors)
INTRAMEDULLARY (5%-10% of all spinal tumors)
Metastasis
Extension of primary bone tumors
INTRADURAL-EXTRAMEDULLARY (30% of all spinal tumors)
gliomas
Generalmente divisi in base alla localizzazione si tratta di una generalizzazione perche’: un a stessa patologia puo’ risiedere in due comparti contemporaneamente opure due lesioni con patologia identica alla base possono insorgere in comparti differenti
Cmq utile
Meningiomas and neurinoma
Leptomeningeal dissemination

3. Sag T1-w fat suppression post contrast
MRI gold standard! TR TE
FOV
Matrix
Sag T2-w
3500
120
275
304X225
Sag T1-w
400 10
Sag T2 STIR
2500 (TI=170) 70
256X190
Ax T2-w
4500
230
288X200
Ax T1-w
541 15
225
304X212
Sag T1-w fat suppression post contrast
Ax T1-w post contrast
In tutto l’ambito della patologia spinale la patolgoia tumorale è quella in cui l’rm has the msot impact
Allo stato attuale è la modality of choice
Estensione localizzazione e struttura interna della massa

4. 1. INTRAMEDULLARY TUMORS
EPIDURAL FAT
SUBDURAL SPACE
SEPTUM POSTICUM
DURA MATER
ARACHNOID
DURAL NERVE ROOT SLEEVE
DENTICULATE LIGAMENT
SPINAL CORD AND PIA
JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

5. Summary
Three general characteristics: 1)they tend to enlarge the cord either focally or diffusely 2) on T2-w they produce high signal intensity 3) They enhance

6. 1.a. SPINAL CORD ASTROCYTOMA
From astrocytes
Most WHO I-II
Most common in CHILDREN
80-90%
low grade
Pilocitic
Fibrillary
20% intraspinal neoplasms
60% primary spinal cord tumors
Il potenziale maligno è piu’ basso rispetto agli astrocitomi cerebrali e la grandezza del tumore non riflette la malignità
subarachnoid dissemination may occur!
Association with abnormalities of chromosome 17p (TP53 gene)

7. Demographic features:
General features
A) Age: two peaks: ys
Demographic features:
B) Gender: M > F (1,3:1);
no gender difference in children!
BEST DIAGNOSTIC CLUE
Enhancing infiltrating cord mass in child
No capsule or cleavage
LOCATION
Thoracic >Cervical > Lumbar
SIZE
Usually 1-3 cm, less than 4 segments
MORPHOLOGY
fusiform expansion of cord, eccentric , posterior
Risk in patients with Neurofibromatosis (NF1 and NF2)

8. b) 40% cysts and syringohydromyelia
MR Findings
T1-w:
a) Cord expansion
Usually < 4 segments
Occasionally multisegmental (holocord more common with pilocytic astrocytomas)
b) 40% cysts and syringohydromyelia
c) Solid portion (hypo-isointense)
CORD EXPANSION
From Rossi et al 2007

9. b) Not as hyperintense if cysts/syrinx
MR Findings
T2-w:
HYPERINTENSE LESION
a) Hyperintense
b) Not as hyperintense if cysts/syrinx
T2* GRE:
Rarely hemorrhagic
CYSTS
From Rossi et al 2007

10. RARELY HEMORRAGE CYSTS
Ci possono essere cisti intratumorali o rostrali e caudali, queste ultime tendono ad essere benigne , possono essere proteinacee o emorragiche ma in genere non hanno cellule tumorali per cui non prendono contrasto e per questo non vanno escisse ma drenate
Mentre cisti intratumorali prendono contyrasto
Sono tumori infiltranti molto difficili spesso non resecabili

11. a) enhancement T1-w post-contrast: MODERATE C.E. ALWAYS!!
Enghancement è il general rule anche se spesso non è granche’ rispetto all’intensità di enhancement dell’ependimoma
From Rossi et al 2007

12. DIFFERENTIAL DIAGNOSIS INTRAMEDULLARY HEMANGIOBLASTOMA
SARCOIDOSIS
INTRAMEDULLARY
EPENDYMOMA
INTRAMEDULLARY HEMANGIOBLASTOMA
From Baker et al 1999

13. ARNOLD-CHIARI I MALFORMATION
SYRINGOMYELIA
Dd con siringhe benigne complesse per margini indistinit segnale disomogeneo non sempre simile al CSF sempre fare contradsto in una siringa a meno che non sia una chiari I in cui lìetiologia della siringa è ovvia

14. 1.b. SPINAL CORD EPENDYMOMA
Cellular ependymomas
(lining spinal cord central canal)
Myxopapillary ependymomas
(from ependymal cells of filum terminale)

15. CELLULAR EPENDYMOMA Neoplasm of ependyma lining
spinal cord central canal
Associated with:
ESA
Superficial siderosis
NF deletion or traslocation chr 22
Most common in ADULT (60%) in lower spine
[ependymoma: 4% of all primary CNS tumors]
2nd most common
in CHILDREN

16. Demographic features:
General features
A) Age: 40 yrs
Demographic features:
B) Gender: M:F=3:1
C) Ethnicity: non racial predilection
BEST DIAGNOSTIC CLUE
Circumscribed, enhancing cord mass with hemorrhage
LOCATION
Conus> Cervico-Thoracic
SIZE
Multisegmental (typically 3-4 segments)
MORPHOLOGY
Well-circumscribed, symmetric cord expansion, occasional exophytic component
Piu’ focali !!! Anche se possono essere estesi per piu’ segmenti
Nonstante non siano capsulati sono circoscritti non infiltranti e benigni istologicamente in alcuni casi tot resecabili

17. Radiological features ISO- HYPOINTENSE LESION
MR Findings
ISO- HYPOINTENSE LESION
T1-w:
Iso- or slightly hypointense to spinal cord
Hemorrhage

18. Radiological features
MR Findings
HEMOSIDERIN
“CAP SIGN”
T2-w:
a)Hyperintense
b) Polar (rostral or caudal) or intratumoral cysts
(50-90%)
Hemosiderin
“Cap sign”
c) Syrinx
d) Focal hypointensity
e) Surrounding cord oedema

19. Radiological features
MR Findings
HEMOSIDERIN
“CAP SIGN”
STIR:
a) Hyperintense
C.E.
T1-w post-contrast:
a) Intense,well-delineated homogeneous c.e. (50%)
b) Nodular, peripheral, heterogeneous c.e.
c) Minimal or no c.e. rare

20. Astrocytoma Ependymoma
cervical
conus
Eccentric posterior
central -
hemorragic
hyercellularity
Infiltrative (poor borders)
Pseudocapsule (plane)

21. MYXOPAPILLARY EPENDYMOMA
from ependymal cells of filum terminale
27-30% of all ependymomas
90% of all filum terminale tumors

22. Radiological features
MR Findings
T1-w:
Usually Isointense with cord
Hyperintensity (accumulation of mucin!)
T2-w:
Hyperintense
Hypointensity at tumor margins (hemosiderin)
STIR:
a) Hyperintense
T1-w post-contrast:
Intense c.e.
From Rossi et al 2007

23. INTENSE C.E. From Rossi et al 2007
Per propensione per cono possono essere sia intramid che extramidollari intradurali
From Rossi et al 2007

24. DIFFERENTIAL DIAGNOSIS ARTERO-VENOUS FISTOLA
Cord often normal/small, gliotic talora ingrandito sa STASI, EDEMA, poi ischemia
focal nodule absent, dorsal veins prominent
M y insidious onset of lower extremities weakness

25. Isointense with cord on T1-w and T2-w
SPINAL MENINGIOMA
From Rossi et al 2007
Isointense with cord on T1-w and T2-w
More common in thoracic and cervical spine (conus and filum location unusual)
Hemorrhage uncommon
Bony changes rare

26. 1.c. SPINAL CORD HEMANGIOBLASTOMA
Capillary rich neoplasms
WHO I
1-5% of all spinal cord neoplasms:
75% sporadic
25% associated with von Hippel-Lindau (VHL)
Lesioni vascolarizzate costiutito da denso network di capillari e canali vascolari
Autosomal dominant (chromosome 3p)
Cerebellar hemangioblastomas, retinal angiomas, pheochromocytoma, renal cell carcinoma, angiomatous or cystic lesions of kidneys, pancreas and epididymis

27. Demographic features:
General features
A) Age: mean age at presentation 30 ys
Demographic features:
B) Gender: M = F
BEST DIAGNOSTIC CLUE
Intramedullary mass with serpentine “flow voids”
LOCATION
Thoracic > Cervical
Subpial
(posterior, rarely anterior)
often associated with intraspinal cysts
SIZE
Few mm to several cm
MORPHOLOGY
Round, well-defined margins

28. Radiological features
MR Findings
T1-w:
Small:
Large:
ISO-
HYPOINTENSE LESION +
SYRINX
Isointense
Cyst!!
(> 50%)
hypo- isointense
“flow voids” (lesion > 2,5 cm)

29. Radiological features
MR Findings
T2-w:
Uniformly hyperintense (small lesions)
mixed hyperintense
(hemorrhage common)
+/- peritumoral oedema
Syrinx fluid
HYPERINTENSE LESION +
CYST

30. Radiological features SUBPIAL NODULE ON THE DORSAL SURFACE
MR Findings
T1-w post contrast:
Subpial nodule
Well-demarcated, intense, homogeneous C.E.
(often on surface of dorsal cord)
Cyst/
Syrinx: no c.e.
Hannpo quindi componente cisticA E COMPONENTE SOLIDA CHE ENANHA
SUBPIAL NODULE ON THE DORSAL SURFACE

31. Possono esser multiple ed eccentriche e quindi apparire come extramidollarti

32. CAVERNOUS MALFORMATION
ARTERIOVENOUS
MALFORMATION (AVM)
CAVERNOUS MALFORMATION
Pattern of prior hemorrhage, hemosiderin rim
Minimal c.e.
AVM una delle poche soddisfaz del neuroradiologo visto che si tratano bene
AC simili all’encefalo
Cord often normal/small, gliotic
focal nodule absent, dorsal veins prominent
M y insidious onset of lower extremities weakness
From Chabert E et al. J Neuroradiol 1999; 26:

33. Ependymoma metastasis Astrocytoma mass centrally located
Metastasi arrivano per disseminazione ematogena oppure per crescita lungo gli spazi di v-R per disseminazione liquorale
Ricordiamo che metastatizzano allo spine oligodendro, gbl, linfoma melanoma e oltre a lung breast e gastric
Crowley, Neurosurgery 2008
not hypervascular;
peritumoral oedema common
Renal cell carcinoma

34. 1)they tend to enlarge the cord either focally or diffusely 2) on T2-w they produce high signal intensity 3) They enhance
In relatà ci sono caratteristiche precipue di ognuno di loro, ma il punto non è distinguerle istopatologicamente bensi’ nella DD
Non alla enlarged cords are neoplasms

35. Symptoms include pain, weakness and muscle atrophy
Demylinating diseases
Infections
Inflammation
Multiple Sclerosis
ADEM
Cord swelling from extramedullary infectious process (as meningitis producing vascular compromise)
AIDS
Sarcoid
LES
Syringohydromyelia
Vascular lesions
Causes of subacute necrotizing myelopahty
Hemorrage
Acute infarction
Venous hypertension
Cavernous angioma
Arterovenous malformation
ADEM
Devic, MS
HZV,Rubeola, Mononucleosis, mumps
Toxo, TBC
Lupus
Spinal dural AVM
Non alla enlarged cords are neoplasms molte cause infiammatorie e demilinizzanti possono allargarlo, quindi specialmente in pz giovani cobn sintomi acuti o subacuti nel dubbio fare RM encefalo nel caso ci siano elsioni addizionali che indirizzano.
In piu’ un consiglio è che si puo’ suggerire l’ipotesi istopatologica ma talora è difficle o impossobile , l’aspetto piu’ importante è identificare che lo spinal cord is abnormal

36. 2. INTRADURAL EXTRAMEDULLARY TUMORS
EPIDURAL FAT
SUBDURAL SPACE
SEPTUM POSTICUM
DURA MATER
ARACHNOID
DURAL NERVE ROOT SLEEVE
DENTICULATE LIGAMENT
SPINAL CORD AND PIA
JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

37. Arise from single nerve, from one side Typically dorsal root
Neurofibroma
Schwannoma, Neurinoma
Arise from single nerve, from one side
Typically dorsal root
Schwann cells
Do NOT envelope the adiacent nerve root
Lobulated
Single
Schwann cells and fibroblasts between dispersed nerve fibers, mps matrix
Spreads apart axons
DO envelope the adiacent nerve root
Fusiform
Multiple
Incapsulati i primi, ben circoscritti ma non incapsulati i secondi ,
nei secondi l’elemento cospicuo è il collagene
hanno poca propensione a emorragia , modificaz vascolari o grasse a differenza dei primi

38. Neoplasm of nerve sheath in PNS
2.a. SCHWANNOMA
Neoplasm of nerve sheath in PNS
Most common intradural extramedullary mass
30% primary spinal tumors
WHO grade I
Le lesioni solitarie sono in genere schwannomi
Mostly sporadic (inactivation of NF2 gene)
Association with NF2, bilateral multiple

39. Demographic features:
General features
A) Age: 40 ys
Demographic features:
B) Gender: M = F
C) Ethnicity: no racial predilection
BEST DIAGNOSTIC CLUE
Well-circumscribed, “dumbbell” shaped, enhancing spinal mass
LOCATION
Thoracic > Cervical = Lumbar
70-75% intradural extramedullary
15% completely extradural
15% “dumbbell” (both intra- and extradural)
SIZE
Few mm
Giant schwannoma: > 2 vertebral segments
MORPHOLOGY
Round, lobulated
Frequentemente associati a emorragia, camb vascolari, cisti e degeneraz grassa

40. Radiological features HYPERINTENSE LESION WITH TARGET SIGN
T1-w:
Isointense
ISOINTENSE LESION
T2-w:
hyperintense
45%cyst 10% hemorrage
“Target sign”
Target sign alto segnale periferico e basso segnale al centro PER AREE piu’ dense di collagene e cellule di schwann è un aspetto di benignità
Formae è allagrato e si vede il tumore passare attraverso
HYPERINTENSE LESION WITH TARGET SIGN

41. Radiological features
T1-w post-contrast:
Intense c.e.
(uniform or peripheral)
Ottimo dettaglio della compressione del midollom
C.E.

42. Localized, diffuse or plexiform neoplasm of nerve sheath
2.b. NEUROFIBROMA
Localized, diffuse or plexiform neoplasm of nerve sheath
WHO grade I
WHO grade III/IV
5% of all benign soft tissue tumors
Association with neurofibromatosis 1 (NF1)
13-65% have spinal neurofibromas

43. Quando hanno un’apparenza diffusa multinodualre si definiscono plessiformi

44. HYPOINTENSE SEPTATIONS
ISOINTENSE
LESION
HYPERINTENSE
LESION +
HYPOINTENSE SEPTATIONS
C.E.

45. DIFFERENTIAL DIAGNOSIS
CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (CIDP)
Polineuropatia sensitivo motoria causata da ripeutiti successivi episodi di demilinizzazione e rimeilinizzazione con un aspeto a cipolla del nervo
Similissima ma non c’e’ il contesto di NF1
Pathology
Hallmarks of CIDP: Enlarged nerves with "onion bulb" formations, demyelination
Clinical Issues
Mixed sensorimotor neuropathy
Symmetric proximal and distal weakness, sensory loss
Clinical course: Chronic progressive, step-wise progressive, or recurrent behavior
T1WI C+: Mild to moderate nerve enhancement cauda equina

46. SECOND MOST COMMON INTRADURAL EXTRAMEDULLARY TUMOR
2.c. MENINGIOMA
Origin from arachnoid
Attached to the dura mater for infiltration
SECOND MOST COMMON INTRADURAL EXTRAMEDULLARY TUMOR
> 25% WHO I
Genetic predisposition in women!
Almost all have chromosome 22 abnormalities
Association with neurofibromatosis 2 (NF2)
Meningothelial
Fibroblastic
Psammomatous
angiomatous
Solitary

47. Demographic features:
General features
Age: yrs
Demographic features:
B) Gender: F:M=4:1 (>70-80% female!)
BEST DIAGNOSTIC CLUE
Enhancing intradural extramedullary mass with “dural tail”
LOCATION
Thoracic (80%)>> Cervical (16%) > Lumbar (4%)
Typical intradural extramedullary
Posterolateral (anterior cervical)
SIZE
Variable
MORPHOLOGY
Typically round with dural attachment, Ca ++ < 5%

48. Radiological features ISOINTENSE LESION WITH CALCIFICATION
MR Findings
Ben circostritti capsulati
Originano dal legamento denticolato e generalmente sono sottodurali e possonmo comprimere il midollo
T1-w:
Isointense
Iso- hyperintense
Hypointense (calcification)
T2-w:

49. Radiological features
C.E.
T1-w post-contrast:
Prominent c.e.
May see enhancing “dural tail”

50. Differential diagnosis
NEURINOMA
More anterior
Very hyperintense on T2-w
with Target sign
Cystic change, hemorrhage more common
No dural attachment
more frequent enlargment of neural foramen

51. DROP METASTASIS
Margini irregolari del midollo o noduli lungo le radici le M pilai posso dare enhancement lineare lungo il cord DD infezioni o infiammz tipo sarcoidosi il CSF puo’ esser piu’ iso che ipo
Fare RM encefalo
Leucemie e linfomi son i preferenziali di parameningeal metastasis

52. 3. EXTRADURAL TUMORS DURA MATER ARACHNOID SPINAL CORD AND PIA
EPIDURAL FAT
SUBDURAL SPACE
SEPTUM POSTICUM
DURA MATER
ARACHNOID
DURAL NERVE ROOT SLEEVE
DENTICULATE LIGAMENT
SPINAL CORD AND PIA
JUNCTION OF DURAL SLEEVE AND SPINAL NERVE PERINEURIUM

53. Female (breast and lung) Male (prostate and lung)
METASTASIS
LYMPHOMA
Female (breast and lung)
Male (prostate and lung)
Thoracic (70%)> lumbar (20%) > cervical (10%)
Ricordiamo che metastatizzano allo spine oligodendro, gbl, linfoma melanoma e oltre a lung breast e gastric
From Thurner et al.

54. DROP METASTASIS

55. DIFFERENTIAL DIAGNOSIS
ANGIOMIOLIPOMA

56. DIFFERENTIAL DIAGNOSIS
GRANULOMATOSIS

57. INTRADURAL ARACHNOID CYST
EPIDURAL EMATOMA

58. Grazie per l’attenzione